For patients with soft tissue sarcoma in an extremity, the outcome is thought to be poor if lymph node metastasis develops. The purpose of this study was to examine the impact of lymphatic involvement from soft tissue sarcoma on patient survival. Thirty-nine (3.7%) of 1066 patients who had surgery for soft tissue sarcoma in an extremity had lymph node metastases develop. Three (20%) of 15 patients with epithelioid sarcoma, four (19%) of 21 patients with rhabdomyosarcoma, two (11.1%) of 18 patients with clear cell sarcoma, and two (11.1%) of 18 patients with angiosarcoma had lymphatic involvement. Thirty patients who had resection of involved lymph nodes had an estimated 5-year survival of 57%, whereas nine patients treated without surgery all died within 30 months. An estimated 4-year survival of 71% for patients with isolated lymph node metastases was significantly better than 21% for patients with synchronous systemic and lymph node involvement. There was no difference in outcome for patients with isolated lymphatic involvement compared with patients with American Joint Committee on Cancer Stage III extremity sarcomas. These results suggest that long-term survival is possible after surgical resection of lymphatic metastases from soft tissue sarcoma. The American Joint Committee on Cancer should consider separating isolated nodal metastases from systemic involvement in patients with Stage IV sarcoma.